The long-term objectives of this project are to contribute to an understanding of the role of glycolipids in cell physiology. The known dramatic effects of testosterone on the kidney glycolipids of the mouse will be utilized to investigate regulation of glycolipid metabolism. Quantitative high performance liquid chromatography, radioisotope labelling studies and analysis of synthetic and degradative enzymes will be employed. The morphological distribution of glycolipids within the kidney will be investigated by microdissection and immunohistochemical methods. Cell fractionation combined with high pressure liquid chromatographic analysis will determine the localization of the glycolipid within the cell. The urinary excretion of glycolipids will be measured in prepubescent normal and Fabry's disease affected individuals.